A 90-year-old man presented with a 6-month history of swelling and redness of his nose. He had persistent erythematous macules, plaques, and partially confluent nodules with irregular borders developing on his nose over the past 6-month period. He was initially seen by his dermatologist and treated with antibiotics and steroids, but with no major improvement. Physical examination revealed large, infiltrative, edematous, erythematous plaques and rare nodules, with superficial telangiectasia, but was negative for pathologic lymphadenopathy or organomegaly. Routine laboratory analysis was normal. A punch biopsy of the nose lesion was performed, revealing atypical proliferative cells infiltrating the skin in many areas, with a poorly vasoformative pattern. Immunohistochemical stain was positive for CD31.
Rhinophyma (answer A) is a benign condition with development of a large, bulbous granulomatous infiltration, commonly due to untreated rosacea. The clinical scenario described here strongly mimics rhinophyma to the point that the patient was initially given steroids and antibiotics; however, he did not respond to that treatment. Kaposiform hemangioendothelioma (answer B) is an aggressive endothelial-derived spindle cell neoplasm that occurs nearly exclusively during childhood or teenage years. Histology would have revealed a lack of cytologic atypia and absence of infiltrative anastomosing channels. Spindle cell sarcoma (answer D) may resemble poorly differentiated angiosarcoma; however, the location and histology makes this diagnosis unlikely. Nodular amelanotic melanoma (answer E) may present similarly, but the clinical picture and immunohistochemical markers (S100, HMB-45) would help in establishing the diagnosis.
Angiosarcomas (correct answer, C) are rare tumors accounting for less than 2% of all sarcomas. Skin and superficial soft tissues are the most commonly involved sites. If lymph nodes are involved or there are systemic metastases, the 5-year survival rate can be as low as 10% to 35%. Angiosarcoma of the skin more commonly involves the face and scalp of the elderly population. Although no known genetic defects or chromosomal abnormalities are implicated in the etiology, possible causative factors proposed include a long history of exposure to solar and/or chronic ultraviolet light, radiation, vinyl chloride, arsenic, insecticides, androgenic steroids, and thorium dioxide.
The clinical presentation is usually characterized by erythematous plaques expanding rapidly over the skin, forming blue-black nodules that often ulcerate. Facial edema with cellulitis or erysipelas can occur. Angiosarcoma of the nose can mimic different conditions, including arteriovenous malformations, nodular melanoma, lymphoma, sarcoidosis, or facial granuloma. Angiosarcoma developing on rhinophyma has been reported.[4,5] Treatment options include wide resection in localized neoplasms, radiation therapy, or chemotherapy for surgically unresectable cases.[2,6] Nasal angiosarcoma can have a better prognosis due to the lower-grade nature and shorter interval between diagnosis and treatment; however, there are few reports that accurately describe the survival rates.
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